Study Questions:

What are the clinical features, management, and long-term outcomes of young patients with the Brugada syndrome who underwent implantable cardioverter-defibrillator (ICD) implantation?

Methods:

A prospective registry was queried. Study inclusion criteria were patients ages ≤20 years and either: spontaneous or drug-induced Brugada type I electrocardiographic (ECG) pattern, who were followed for >6 months post-device implantation.

Results:

From a total of 1,638 patients, 35 were aged ≤20 years and had an ICD. The mean age at ICD implant was 13.9 ± 6.2 years. Among them, 29% presented with aborted sudden cardiac death, and 63% with syncope. During a mean follow-up period of 88 months, sustained ventricular arrhythmias were treated by the ICD in nine (26%), including shocks in eight patients (23%) and anti-tachycardia pacing in one patient (3%). Three patients (9%) died in an electrical storm. Seven patients (20%) experienced inappropriate shocks, and five patients (14%) had device-related complications. Aborted sudden cardiac death and spontaneous type I ECG were identified as independent predictors of appropriate shock occurrence.

Conclusions:

ICD is an effective treatment in young patients with symptomatic Brugada syndrome. Appropriate shocks were significantly associated with previous aborted sudden cardiac death and spontaneous type I ECGs.

Perspective:

An ICD is the only therapy effective in extending life in patients with Brugada syndrome, although quinidine may reduce the rate of appropriate therapy in patients who survived a ventricular arrhythmia storm. Most patients who are diagnosed with Brugada syndrome are adult men, usually in their third or fourth decade in life. The present study discusses outcomes in ICD patients with Brugada who are diagnosed before the age of 20 following an aborted cardiac arrest or “severe” syncope and a spontaneous occurrence of Brugada type ECG pattern. Interestingly, in the pediatric cohort, the number of boys and girls was about the same. In this cohort, the rate of appropriate therapy appears to be as high as in the older population. Inappropriate therapy is an important issue in both age groups. The rate of inappropriate therapy decreased in recent years, likely due to more restrictive device programming (i.e., single ventricular fibrillation therapy zone starting at 240 bpm with a 30-40 second delay).